Anomalous pancreaticobiliary duct union associated with bile duct carcinoma

An abnormal high union of the common bile duct and the main pancreatic duct, without accompanying cystic dilatation of the bile ducts, is a rare occurrence. A case of obstructive jaundice due to bile duct carcinoma in connection with this anomaly is reported.     

A rare ring-shaped anomaly of the main pancreatic duct accompanying a branch duct IPMN

The patient was a 74-year-old female. Screening computed tomography for examination of the abdomen showed a cystic mass in the pancreatic body. Close investigation using endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography revealed a very rare finding: the main pancreatic duct bifurcated at the pancreatic body, and these two ducts converged at the caudal side. A multilocular cystic mass in the pancreatic body and mucus discharge from the orifice of major papilla were observed. There was no protruded lesion in the main pancreatic duct. No findings suggested apparent malignancy. The patient was diagnosed as having hyperplastic intraductal papillary mucinous neoplasm of branch type showing a ring-shaped pancreatic duct, and was placed under follow-up.     

Serous cystadenoma of the pancreas communicating with a pancreatic duct

Summary Conclusion To differentiate serous cystadenoma from other cystic lesions communicating with the pancreatic duct, careful radiological examination is necessary. Background Communication between the cystic cavity and the pancreatic duct in an ordinary serous cystadenoma is uncommon, although it is not uncommon in other cystic lesions, such as pseudocyst, mucinous cystadenoma/adenocarcinoma, and intraductal papillary tumor. Methods A serous cystadenoma of the pancreas communicating with main pancreatic duct in a 76-yr-old male is reported. Results The communication was preoperatively demonstrated by endoscopic retrograde cholangiopancreatography and confirmed by histopathological examination of the resected specimen.     

Double cancer of the cystic duct and gallbladder associated with low junction of the cystic duct

We report a case of double cancer of the cystic duct and gallbladder associated with low junction of the cystic duct. A 73-year-old woman was admitted to the hospital complaining of upper abdominal pain. Endoscopic retrograde cholangiography showed a stenotic lesion in the lower common bile duct and no visualization of the cystic duct or gallbladder. Enhanced computed tomography revealed a heterogeneously enhanced tumorous lesion around the lower bile duct in the pancreatic head. A diagnosis of cancer arising from the cystic duct that entered the lower part of the common hepatic duct was made by intraductal ultrasonography, which showed an intraluminal protruding lesion in the cystic duct. Isolated gallbladder cancer was also diagnosed, by abdominal computed tomography. She underwent pancreaticoduodenectomy with dissection of regional lymph nodes. Histological examination revealed moderately differentiated adenocarcinoma of the cystic duct and well-differentiated adenocarcinoma of the gallbladder. Double cancer of the cystic duct and gallbladder is extremely rare, and this case also suggests a relationship between a low junction of the cystic duct and neoplasm in the biliary tract.     

A giant retention cyst of the pancreas (cystic dilatation of dorsal pancreatic duct) associated with pancreas divisum

We describe a rare case of pancreas divisum associated with a giant retention cyst (cystic dilatation of the dorsal pancreatic duct), presumably formed following obstruction of the minor papilla. The patient was treated by pancreatico(cysto)jejunostomy. A 50-year-old man was admitted with complaints of increasing upper abdominal distension and body weight loss. There was no previous history of pancreatitis, gallstones, drinking, or abdominal injury. An elastic-hard tumor-like resistance was palpable in the upper abdomen. Computed tomography and ultrasound (US) examinations revealed a giant cystic lesion expanding from the pancreas head to the tail. Endoscopic retrograde cholangiopancreatography findings showed a looping pancreatic duct which drained only the head and uncinate process of the pancreas to the main papilla. A US-guided puncture to the cystic lesion revealed that the lesion continued to the main pancreatic duct in the tail of pancreas. The lesion was connected to a small cystic lesion, which was located inside the minor papilla, and ended there. The amylase level in liquid aspirated from the cyst was 37 869 IU/l, and the result of cytological examination of the liquid showed class II. A pancreatico(cysto)jejunostomy was performed, with the diagnosis being pancreas divisum associated with a retention cyst following obstruction of the minor papilla. The histological findings of a specimen from the cyst wall revealed that the wall was a pancreatic duct covered with mildly inflammatory duct epithelium; there was no evidence of neoplasm. The patient is currently well, and a CT examination 2 years after the operation showed disappearance of the cyst and normal appearance of the whole pancreas. Received: April 24, 2001 / Accepted: September 14, 2001     

Primary hydatid cyst of the pancreas related to main pancreatic duct. A case report

A case of primary hydatid cyst of the pancreas related to the main pancreatic duct is presented. Abdominal ultrasound and computed tomography revealed a cyst at the tail of the pancreas. Endoscopic retrograde cholangiopancreatography showed dilatation of the distal part of the pancreatic duct related to the cystic cavity. While the diagnosis of the pancreatic pseudocyst was established preoperatively, the hydatid cyst was demonstrated at laparotomy and then the final decision of cystogastrostomy was employed. Following the uneventful postoperative period, the patient was discharged on the 8th postoperative day.     

Macrocystic type of serous cystadenoma with a communication between the cyst and pancreatic duct

A 42-year-old woman with a cystic lesion in the head of the pancreas was evaluated by using abdominal ultrasonography, a computed tomographic scan, magnetic resonance imaging and endoscopic retrograde pancreatography. Multiple cystic lesions, 5 cm in diameter, which had papillary protrusion inside the cyst in the head of the pancreas and had the communication between the cysts and pancreatic duct, were determined. Pylorus-preserving pancreaticoduodenectomy was performed under the diagnosis of mucinous cystic neoplasm of the pancreas. Although the cut surface of the tumor showed a macrocystic tumor of 3 cm in diameter, part of the cyst wall was cavernous. A histopathological examination showed single-layered cuboidal cells, which lead to the diagnosis as being serous cystadenoma of the pancreas. Serous cystadenoma is a rare, almost benign pancreatic tumor. The macrocystic subtype of serous cystadenoma is even more rare. We describe a patient who had this macrocystic subtype of serous cystadenoma with a communication between the cyst and pancreatic duct. This case illustrates the difficulty in the diagnosis of cystic lesions in the pancreas, and might support the single category of cystic lesions of the pancreas.     

Risk factors for pancreatitis in patients with anomalous union of pancreatobiliary duct

BACKGROUND/AIMS: The clinical features of pancreatitis in patients with an anomalous union of the pancreatobiliary duct (AUPBD) are unclear. This study analyzed the clinical features, pathogenic mechanisms, risk factors, and clinical courses after treatment in AUPBD patients. METHODOLOGY: The medical records and cholangiopancreatograms of 58 patients with AUPBD and a choledochal cyst diagnosed between 1982 and 2001 were retrospectively reviewed. RESULTS: Sixteen (27.6%) out of the 58 AUPBD patients experienced pancreatitis. The incidence of pancreatitis was significantly higher in those patients with a long (>21mm) and wide (>5mm) common channel, a wide diameter of the proximal pancreatic duct (>2.5mm), the presence of a filling defect in the common channel, and the presence of a pancreatic duct anomaly (p<0.05). A bile duct diversion from the pancreatic duct by a choledochal cyst excision prevented the recurrence of pancreatitis in most cases. A duodenopancreatectomy was required in some cases. CONCLUSIONS: The morphological characteristics of the common channel and pancreatic duct contribute to the development pancreatitis in AUPBD patients and bile reflux into the pancreatic duct might be the main mechanism of pancreatitis in these patients.     

Association of gallbladder carcinoma and anomalous pancreaticobiliary ductal union

A total of 96 patients with gallbladder carcinoma in whom direct cholangiography clearly opacified the pancreaticobiliary ductal union and the common channel, and 65 patients with an anomalous union of these two duct systems at a distance greater than 15 mm from the papilla of Vater (normally less than 4.6 +/- 2.2 mm, mean +/- SD) were studied. It was found that this anomalous ductal union occurred in 16.7% of the patients with gallbladder carcinoma in comparison with an incidence of 2.8% among 641 consecutive patients with various hepatobiliary and pancreatic diseases studied by endoscopic retrograde cholangiopancreatography who did not have gallbladder carcinoma. It was also found that gallbladder carcinoma occurred in 24.6% of the 65 cases of anomalous ductal union in comparison with a 1.9% incidence of this cancer among 635 consecutive patients similarly studied and found to have normal ductal union (p less than 0.001). Thus, a close etiologic association was suggested between this anomaly in the terminal segment of the biliary tract and gallbladder carcinoma. Of the 65 patients with anomalous ductal union, 50 had the so-called congenital cystic dilatation of the common bile duct and 15 did not. Five of the 50 (10%) and 11 of the 15 (73.3%) had gallbladder carcinoma (p less than 0.01), and this carcinoma seems to be related to anomalous ductal union rather than to cystic dilatation of the common bile duct. As a tumorigenic factor in this anomaly, regurgitation of pancreatic juice has been stressed.     

A case of gallbladder adenomyomatosis with pancreaticobiliary maljunction and an anomaly of the cystic duct joined the common channel

A 46-yr-old woman was admitted to our hospital with mild epigastric pain. Ultrasonography and computed tomography revealed an extremely thickened gallbladder wall. Endoscopic retrograde cholangiopancreatography demonstrated that the main pancreatic duct joined the nondilated common bile duct at the outer point of the duodenal wall (P-C type of pancreaticobiliary maljunction), and the cystic duct joined the common channel directly. The intraoperative amylase levels of the bile juices both in the common bile duct and the cystic duct were high. A cholecystectomy was performed. The wall of the gallbladder was markedly thick, yellowish, elastic, and soft. Histologically, Rokitansky-Aschoff sinus proliferation, hypertrophy of smooth muscles, and fibrosis were seen. The diagnosis was a generalized type of adenomyomatosis. The pathogenesis of the adenomyomatosis was believed to result from chronic stimulation as a result of pancreatic juice reflux. The etiology of this unusual type of junction was considered to be the result of the combination of pancreaticobiliary maljunction and an anomaly of lower junction of the cystic duct.     

Double duct-to-mucosa pancreaticojejunostomy for bifid pancreatic duct

We report a pancreaticojejunostomy with double duct-to-mucosa anastomotic technique after pyloruspreserving pancreaticoduodenectomy for chronic pancreatitis with bifid pancreatic duct. A 49-year-old Japanese man was diagnosed preoperatively as having chronic pancreatitis with common bile duct stricture and pseudocyst of the pancreatic head. In a pancreaticoduodenectomy, the main pancreatic duct (7mm in diameter) and a secondary pancreatic duct (4mm in diameter) were identified intraoperatively at the transected surface. Pancreatography showed the main pancreatic duct as well as thesecondary pancreatic duct that drained the remaining dorsal pancreas, allowing us to diagnose bifid pancreatic duct. The pancreaticojejunostomy was performed in an end-to-side manner to create double duct-to-mucosa anastomoses and to approximate the pancreatic parenchyma and jejunal seromuscular layers. Although bifid pancreatic duct is a rare anatomical anomaly, it behooves every surgeon who performs pancreatic resections to be aware of this entity and the techniques for dealing with it.     

Aberrant hepatic duct connected with the main pancreatic duct by anomalous pancreato-biliary ductal union: Case report

An aberrant hepatic duct directly connected to the main pancreatic duct with anomalous arrangement of the pancreato-biliary ductal system is reported here, the first report of such a case, to our knowledge. A 53-year-old woman was admitted to our hospital because of cholecystolithiasis with abdominal pain in the right upper quadrant. Endoscopic retrograde cholangiopancreatography (ERCP) showed that an aberrant hepatic duct, which independently drained the right posterior segment of the liver, connected to the main pancreatic duct at a high insertion site distal to the sphincter area of the major papilla. The common bile duct (containing stones), on the other hand, united with the main pancreatic duct in a normal fashion. Cholecystectomy and bile duct lithotomy were performed. The aberrant hepatic duct was separated from the main pancreatic duct just above the junction, and was anastomosed side-by-side to the common hepatic duct. The embryologic development of this lesion is not clear, but is discussed in this report.     

Clinicopathologic study of resected cases of primary carcinoma of the cystic duct

BACKGROUND/AIMS: There have been few reports of primary carcinoma of the cystic duct (CCD) included in advanced cases. The aim of this study was to elucidate the clinical features of resected CCD. METHODOLOGY: Six cases of CCD were diagnosed in which the main carcinomatous component arose from the cystic bile duct, even if these carcinomas were accompanied by invasion beyond the cystic duct. Histopathologic findings (i.e., H.E. staining and cell proliferating potency assessed by ki-67 staining) were compared between the main lesion and invasive lesion of the CCD. RESULTS: Abdominal ultrasonography revealed swelling of the gallbladder in 3 of the 6 patients, but not in the remaining 3, who were later diagnosed as having adenomyomatosis of the gallbladder, dystelectasis due to the carcinomatous infiltration, and atrophic gallbladder, respectively. On computed tomography, 4 of the 6 cases with nodular-type lesions fulfilling Farrar's criteria, the tumors showed contrast enhancement. Direct cholangiography demonstrated unilateral obstruction of the common bile duct in 4 out of the 6 cases. Intraductal ultrasonography revealed CCD in only 1 of the 6 cases. Advanced CCD shows 2 patterns of invasion; the hepatic hilum pattern and the confluence invasive pattern. The hepatic hilum pattern of invasion tends to be associated with a poorer prognosis. Histopathological study revealed papillary and/or well differentiated adenocarcinoma in the cases where the lesion predominantly involved the cystic duct, whereas those lesions which extended beyond the cystic duct were composed of moderate and/or poorly differentiated tubular adenocarcinoma. The latter was associated with a high cellular proliferative activity as assessed by immunocytochemical examination for ki-67. Invasion of the perineural space was often observed in the cases with advanced CCD. CONCLUSIONS: CCD showed the hepatic hilum and/or confluence pattern of invasion when the tumor extended beyond the cystic duct. CCD extending beyond the cystic duct was associated with more aggressive characteristics of the tumors, with perineural infiltration and histopathologic features resembling those of pancreatic cancer. It is concluded that CCDs extending beyond the cystic duct are more aggressive and associated with a poorer prognosis.     

A case of mediastinal pancreatic pseudocyst accompanied by pancreatic pleural effusion]

A 52-year-old man was admitted with epigastric and back pain. Chest X-ray and whole body CT scan revealed left massive pleural effusion and cystic lesion in the posterior mediastinal compartment extending to the pancreatic body via the esophageal hiatus. MRCP showed the communication between the cystic lesion and main pancreatic duct which was irregularly dilated. Thoracentesis revealed sterile bloody fluid with markedly elevated amylase activity of 5,770 IU/l (pancreatic isozyme, 100%) and no malignant cells. From these examinations, a diagnosis of chronic alcohol-related pancreatitis accompanied by mediastinal pancreatic pseudocyst and pancreatic pleural effusion was made. We employed conservative therapy including chest tube drainage followed by operation (main pancreatic duct jejunal side-to-side anastomosis). The clinical course has been uneventful for more than 1 year after discharge.     

Embryology of the pancreatic duct system

BACKGROUND/AIMS: It has been suggested that the distal portion of the dorsal pancreatic duct and the ventral pancreatic duct usually merge into the main pancreatic duct, and the proximal portion of the dorsal pancreatic duct becomes the accessory pancreatic duct. In this study, we investigated the embryology of the accessory pancreatic duct roentgenographically and immunohistochemically. METHODS/RESULTS: The accessory pancreatic duct shows two different patterns in pancreatograms: the long and the short type. The accessory pancreatic duct of the long type forms a straight line and joins the main pancreatic duct at the neck portion of the pancreas. The accessory pancreatic duct of the short type joins the main pancreatic duct near its first inferior branch. Long inferior branches from the accessory pancreatic duct were found in 74.6% with the long type, significantly more often than in the short type (29. 3%). Patency of the long type was (74.5%) significantly greater than in the short type (36.2%). Immunohistochemically, we found the main pancreatic duct between the junction with the accessory pancreatic duct and the neck portion of the autopsy pancreas in the short type was located within the ventral pancreas, characterized by pancreatic polypeptide-rich islets. CONCLUSION: The long type represents a continuation of the main duct of the dorsal primordium. The short type is very likely formed by the proximal main duct of the dorsal primordium and its long inferior branch, with the main duct of the dorsal primordium at the point of connection with the main duct of the ventral primordium being obliterated and replaced by this additional communication.     

Clinical significance of the accessory pancreatic duct

BACKGROUND/AIMS: The accessory pancreatic duct is the smaller and less constant pancreatic duct in comparison with the main pancreatic duct. We investigated the patency of the accessory pancreatic duct and its role in pancreatic pathophysiology. METHODOLOGY: Dye-injection endoscopic retrograde pancreatography was performed in 411 patients. In patients in whom the main pancreatic duct could be selectively cannulated, contrast medium with indigo carmine was injected through the catheter. Excretion of the dye from the minor duodenal papilla was observed endoscopically. RESULTS: Patency of the accessory pancreatic duct was 43% of the 291 control cases. In the 46 patients with acute pancreatitis, 8 (17%) had a patent accessory pancreatic duct. The difference in patency between this group and the normal group was significant (p < 0.01). Especially, patency of the accessory pancreatic duct was only 8% of the 13 patients with acute biliary pancreatitis. In the patients with pancreaticobiliary maljunction, biliary carcinoma occurred in 72% of patients with a nonpatent accessory pancreatic duct, but in contrast, it occurred only in 30% of those with a patent accessory pancreatic duct. This difference was significant (p < 0.05). Lower amylase level in the bile of patients with pancreaticobiliary maljunction with a patent accessory pancreatic duct was frequently observed than those with a nonpatent accessory pancreatic duct. CONCLUSIONS: A patent accessory pancreatic duct may prevent acute pancreatitis by lowering the pressure in the main pancreatic duct. In cases of pancreaticobiliary maljunction with a patent accessory pancreatic duct, the incidence of carcinogenesis of the bile duct might be lower, as the reflux of the pancreatic juice to the bile duct might be reduced by the flow of the pancreatic juice into the duodenum through the accessory pancreatic duct.     

Villous tumors of the Wirsung's duct and pancreatic intraductal adenocarcinoma: interrelation or accidental association?]

Villous neoplasms of the main pancreatic duct are uncommon. Two cases of neoplasm of the main cephalic pancreatic duct in 61- and 42-year-old patients presenting with long standing (10 and 12 years) history of abdominal pain are reported. In both cases, duodenal fistula was present and mucus was observed by endoscopy at the fistula and major papilla levels. Endoscopic retrograde pancreatography showed a stricture of the main pancreatic duct in the pancreatic head. In one case, with incomplete stricture, pancreatic ducts disclosed typical features of chronic obstructive pancreatitis and contained mucus casts. Histologic examination of total and proximal duodenopancreatectomy showed a villous neoplastic pattern with focal malignant changes within the main pancreatic duct. The adjacent pancreatic tissue showed signs of stromal invasion without lymph node or nervous infiltration. Glandular parenchyma was atrophic in the pancreatic body and tail, with extensive fibrosis, and the pancreatic duct depicted signs of nonpapillary hyperplasia. Histochemical study disclosed a predominant sialomucin secretion by villous adenoma and sulfomucin secretion by epithelial cells lining the accessory or main caudal pancreatic ducts. These results lead us to suggest a possible relationship between villous adenoma of ducts and pancreatic adenocarcinoma.     

Carcinoma of the cystic duct

Two patients with carcinoma of the cystic duct presented with obstructive jaundice due to extrinsic compression of the common hepatic duct by the tumor. Sonography and computed tomography showed dilatation of the intrahepatic bile ducts and gallbladder. In one patient, a calculus seen in the gallbladder neck suggested Mirizzi syndrome. In the other, a small soft tissue mass was indistinguishable from a common duct tumor or an enlarged lymph node. In both cases, direct cholangiography demonstrated extrinsic compression and displacement of the common duct with proximal biliary dilatation and nonvisualization of the gallbladder. Carcinoma of the cystic duct should be considered whenever there is evidence of cystic duct obstruction and/or when cholangiography shows extrinsic mass effect on the common duct.     

Acinar cell carcinomas with exophytic growth and intraductal pancreatic duct invasion: peculiar multislice computed tomographic picture

On an ultrasound and multislice computed tomography (MSCT), a 76-year-old woman was found to have a huge mass lesion in the pancreatic head. MSCT showed 8.4 cm well-enhancing exophytic tumor of the pancreatic head which also protruded into the duodenum. A tongue-like protrusion into the main pancreatic duct was depicted. Laboratory data showed elevated carbohydrate antigen 19-9. Whipple’s operation was performed. The pathological diagnosis was acinar cell carcinoma (ACC) originating in the pancreatic head and directly invading through the duodenal wall and the main pancreatic duct, without any lymph node involvement. A peculiar ACC with good enhancement, exophytic picture and tongue-like protrusion into the main pancreatic duct on MSCT is presented.     

Villous adenoma of the main pancreatic duct: a potentially malignant tumor?

This report deals with two cases of villous adenoma of the Wirsung duct. The two patients presented with upper abdominal pain, diarrhea, and weight loss. Duodenal intubation showed a complete failure of exocrine pancreatic function. Ultrasound scan, computed tomography, and endoscopic retrograde cholangiopancreatography disclosed marked dilatation of the head portion of the main pancreatic duct. A proximal duodenopancreatectomy was performed. Pathological examination revealed a papillary polyp expanding the pancreatic head and filling the main duct lumen. Histological pattern consisted of a villous adenoma without any feature of malignant change. Previously reported cases exhibit many similarities to our two cases. The putative likelihood of malignant change and the relationship between villous tumors of the Wirsung duct and of other origin remain unclear.